Spontaneous hepatic haemorrhage after caesarean section in a patient with uraemia and superimposed preeclampsia: a case report

Abstract

Perinatal spontaneous hepatic haemorrhage is a very rare disease affecting pregnant women, particularly those on long-term dialysis, that has a high maternal and infant mortality rate. Most patients experience preeclampsia with haemolysis, elevated liver enzymes and low platelets syndrome. Here, the case of a 35-year-old multigravida patient with known chronic kidney disease and chronic hypertension with uraemia, who developed spontaneous hepatic haemorrhage after caesarean section, is described. The patient experienced sudden massive circulatory failure, but hemodynamics were temporarily stabilized after emergency surgery. Following transfer to the intensive care unit for continued treatment, her blood pressure and haemoglobin level continued to drop. Selective hepatic artery embolization was performed on day 2 after delivery, and her vital signs gradually stabilized. On day 30 after delivery, the patient was discharged in a stable condition. The newborn recovered after therapy in neonatal intensive care for 2 months. The present case suggests that, for perinatal spontaneous hepatic haemorrhage, timely and accurate diagnosis, multidisciplinary management and determining the therapeutic approach according to clinical symptoms are essential.

Introduction

The occurrence of perinatal spontaneous hepatic haemorrhage in pregnant women with uraemia is very rare and is associated with relatively high levels of maternal and child mortality. ¹ Most pregnant patients with spontaneous hepatic haemorrhage have preeclampsia and haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.^2,3 Chronic renal failure is also regarded as a risk factor for hepatic haemorrhage. To date, the pathophysiology of perinatal spontaneous hepatic haemorrhage remains unclear, and its treatment is often controversial. ⁴ Here, a case of perinatal spontaneous hepatic haemorrhage in a multigravida patient with a history of chronic kidney disease, with preeclampsia superimposed on chronic hypertension, is described. Hepatic artery embolization combined with surgery resulted in positive maternal and infant outcomes.

Case report

A 35-year-old multigravida patient was admitted to the maternity ward of Anqing Municipal Hospital, Anqing City, China in November 2021, at 26⁺³ weeks of gestation, due to progressively elevated blood pressure and blurred vision. She had a 3-year history of hypertension, proteinuria and uraemia with haemodialysis treatment. The cause of chronic kidney disease was unclear. It was revealed that, before this pregnancy, the patient was required to take one 30 mg nifedipine tablet, orally, once daily, to regulate blood pressure, and received dialysis twice per week. At 12 weeks of gestation, she was found to have an accidental pregnancy, and was advised to terminate the pregnancy during prenatal examination, due to a history of chronic kidney disease and the need for haemodialysis treatment. However, the patient strongly requested to continue the pregnancy. Following detection of the pregnancy, from 12 to 26 weeks of gestation, she received dialysis five times per week, and from 26 to 30 weeks of gestation, she received dialysis six times per week. During pregnancy, her urine output was about 300 ml daily and her creatinine level was maintained at around 500 µmol/L. She had a history of one caesarean section and three abortions over the previous 17 years, and no history of trauma or liver disease. Her spouse was healthy, and her family history was unremarkable.

On admission, the patient was diagnosed with uraemia and superimposed preeclampsia. She was managed with 0.95 µg/kg/min nitroglycerin by continuous intravenous pumping to control her blood pressure. At 30⁺⁴ weeks of gestation, caesarean section under general anaesthesia was performed because fetal distress was considered. The live male newborn, 1050 g in weight, had an Apgar score of 5 at 15 min and was transferred to neonatal intensive care. During the caesarean section, intraoperative bleeding was 200 ml without iatrogenic injury, such as intraperitoneal compression. The caesarean section went well and the patient was transferred to the postpartum unit.

Approximately 8 h after delivery, physical examination revealed tenderness in the upper abdomen. At approximately 9 h after delivery, the patient gradually descended into a shallow coma, and her skin was pale and cold. Her blood pressure was 60/25 mmHg (with 3.81 µg/kg/min norepinephrine, continuous i.v. pumping), pulse rate was 54 beats/min, and respiratory rate was 40 breaths/min. Vaginal bleeding was within normal limits. Routine blood examination showed the following results: haemoglobin, 68 g/L (pre-surgery, haemoglobin, 106 g/L); and platelet count, 169 × 10⁹/L. Biochemical test results were: aspartate aminotransferase, 27 U/h; alanine aminotransferase, 24 U/h; creatinine, 589 µmol/L; and lactic acid, 9 mmol/L. Blood coagulation function results were: prothrombin time, 12.7 s; activated partial thrombin time, 28.2 s; and fibrinogen, 3.51 g/L. A celiac computed tomography (CT) scan showed perihepatic effusion with hypodensity, and a final diagnosis of spontaneous hepatic haemorrhage was made. The patient suddenly experienced massive circulatory failure, and emergency fluid resuscitation (500 ml succinyl gelatin, i.v. infusion), vasoactive drug support (1.14 µg/kg/min norepinephrine, continuous i.v. pumping), blood transfusion (6 units of red blood cells [RBCs] and 350 ml plasma) and an explorative laparotomy were immediately performed, revealing hemoperitoneum (1000 ml) and a large subcapsular haematoma on the surface of the right lobe of the liver. The central region of the capsule was ruptured, and blood actively flowed from the rupture into the abdominal cavity (Figure 1a). The hemoperitoneum and subcapsular haematoma were removed from the abdominal cavity, and the wound was treated with electrocoagulation. The abdominal cavity was washed with saline solution, and no significant active bleeding was observed (Figure 1b). Two right hepatophrenic plain and omental-bursa drains were left in the abdomen. Following this laparotomy, the patient’s hemodynamic parameters were temporarily stable. Throughout the laparotomy, 6 units of RBCs and 350 ml of plasma were transfused. Postoperative blood samples showed a haemoglobin level of 80 g/dL. The patient was transferred to the intensive care unit (ICU) after surgery to continue monitoring, fluid resuscitation (2000 ml saline solution and 1000 ml succinyl gelatin, i.v. infusion), vasoactive support (0.14 µg/kg/min norepinephrine, continuous i.v. infusion), blood transfusion, invasive mechanical ventilation, continuous renal replacement therapy, parenteral nutrition and antibiotic treatment.

Figure 1.

Post-delivery intraoperative upper abdominal images from a 35-year-old multigravida patient with spontaneous hepatic haemorrhage, showing: (a) spontaneous rupture of the liver capsule (yellow arrow) and liver subcapsular haematoma (blue arrow); and (b) electrocoagulation to stop the bleeding.

After entering the ICU, the patient received 12.5 units of RBCs and 1250 ml of plasma in total, but her blood pressure and haemoglobin levels continued to drop. On day 2 after delivery, the patient exhibited anuria and blood pressure of 108/80 mmHg (with 0.48 µg/kg/min norepinephrine, continuous i.v. pumping). Routine blood examination showed the following results: haemoglobin, 46 g/L and platelet count, 84 × 10⁹/L. Biochemical test results were: aspartate aminotransferase, 3590 U/h; alanine aminotransferase, 3081 U/h; creatinine, 477 µmol/L; and lactic acid, 5 mmol/L. Blood coagulation function results were as follows: prothrombin time, 23 s; activated partial thrombin time, 46.3 s; and fibrinogen, 2.32 g/L. Contrast-enhanced abdominal CT showed that the S6 segment of the liver was enhanced with arterial focus (Figure 2a). Multidisciplinary consultation determined that active hepatic bleeding was still present, and a decision was made to embolize the distal hepatic artery. Percutaneous hepatic arteriography showed that the contrast agent was concentrated, and extravasated pseudoaneurysms were present, in the small vessels of the liver S6 segment (Figure 2b). Consequently, a branch of the right hepatic artery was embolized. Following embolization, no contrast extravasation was observed (Figure 2c), and the patient’s vital signs gradually returned to normal.

Figure 2.

Imaging of the hepatic artery in a 35-year-old multigravida patient with spontaneous hepatic haemorrhage: (a) contrast-enhanced abdominal computed tomography image showing hepatic haemorrhage in liver segment 6 (yellow arrow); (b) hepatic angiography image showing extravasation from the arterial branch of liver segment 6 and microaneurysms (red arrow); and (c) postembolization hepatic angiography image of the right hepatic artery.

On day 6 after delivery, she was taken off the ventilator, and the endotracheal tube was removed. On day 8 after delivery, the patient's clinical symptoms improved, and she was downgraded to the postpartum unit. Subsequent CT showed that the perihepatic haematoma had been absorbed, and no infarction or residual abscess was observed in the liver. While in the postpartum unit, the patient’s urinary volume was approximately 200 ml/day and creatinine level was approximately 600 mmol/L. The patient required dialysis three times per week to maintain homeostasis, and one 30 mg nifedipine tablet, orally, once daily, to help control her blood pressure. On day 30 after delivery, the patient was discharged. The newborn developed well in the neonatal ICU (NICU) and was discharged from the NICU after 2 months, weighing 2100 g.

Ethical approval was not required due to the case report study design. This case report conforms to CARE guidelines, ⁵ and the patient provided written informed consent for the treatment and for publication of the clinical information, including accompanying images.

Discussion

Perinatal spontaneous hepatic haemorrhage may typically occur at any time in the third trimester of pregnancy, and may also occur within 48 h after delivery. The right lobe is the most frequent location of spontaneous hepatic haemorrhage, observed in 75–77% of cases.^6,7 In the present case, postpartum haemorrhage was found in the right lobe of the liver. Maternal and perinatal mortality rates from liver haemorrhage have been reported to be 15% and 41%, respectively, over the last 10 years. ⁴ Due to these extremely high mortality rates, early diagnosis and appropriate management are important for decreasing maternal and fetal mortality in the perinatal period.

To date, the pathophysiology of spontaneous hepatic haemorrhage remains unclear, but most patients have preeclampsia or HELLP syndrome, ⁸ and chronic kidney disease is associated with increased preeclampsia risk. ⁹ Preeclampsia is a complex disorder causing significant multiorgan dysfunction, and can lead to fibrin deposition in the hepatic sinusoids and blood vessels, platelet activation, thrombus formation, vasospasm, and endothelial cell damage. Moreover, increased oxidative stress caused by increased blood levels of toxic metabolites in the ovum or placenta, lipid peroxides in pregnancy with preeclampsia, and decreased maternal antioxidant capacity, may result in vascular endothelial cell damage, leading to vasculopathy. A clear hepatic arteriogram from the present case provides new evidence that vasculopathy may play a primary role in the pathogenesis of spontaneous hepatic haemorrhage during the perinatal period. This may explain why pseudoaneurysms were present in the area of the hepatic haemorrhage in the present case and in previously reported patients. ¹⁰ In addition, bleeding is a common serious complication of end-stage kidney disease. The pathogenesis of bleeding in uraemia is multifactorial, as it includes several aspects, such as the use of heparin during dialysis, platelet dysfunction caused by recurrent and prolonged exposure of blood to the artificial surface of the dialyzer membrane, impaired platelet adhesiveness and altered platelet vessel wall interaction, leading to haemostasis dysfunction. ¹¹

Spontaneous hepatic haemorrhage has a variable clinical presentation, is rapidly progressive, has a low incidence, and diagnosis is often delayed, which is probably the major reason for high rates of perinatal mortality. Some women present with intense epigastric pain, an abdominal mass and rapidly progressive massive circulatory collapse, ¹² as in the present case. Thus, hepatic haemorrhage should be suspected in perinatal patients with preeclampsia/HELLP syndrome who have intense epigastric pain or a sudden fall in blood pressure. Imaging techniques have been used to confirm a suspicion of hepatic haemorrhage. ¹³ For example, ultrasound can be performed at the bedside and is suitable for pregnant patients. Perinatal celiac ultrasonography may be considered as a routine examination procedure for patients with preeclampsia who are receiving haemodialysis. CT is applied for cases in which the patient is hemodynamically unstable, because the CT examination may be completed relatively quickly, and contrast-enhanced abdominal CT may be used as the best non-invasive examination method to further confirm the bleeding vessel within the liver in some patients. In the present case, contrast-enhanced abdominal CT revealed the blood vessel lesion and facilitated transcatheter embolization. Percutaneous hepatic angiography is the gold standard for diagnosing hepatic haemorrhage caused by vasculopathy. ¹⁴

Hepatic haemorrhage in pregnancy requires appropriate and rapid treatment by a multidisciplinary team. In determining treatment, the team must take into account the hemodynamic status of the patient and the integrity of the liver capsule. Treatment for patients who are hemodynamically stable with capsule integrity is mostly conservative. ¹⁵

Hemodynamically unstable patients should be immediately given fluid and blood resuscitation, and surgical treatment.^16–18 However, surgical treatments, such as abdominal packing and liver lobectomy may lead to haemostatic failure, postoperative abdominal infection, liver dysfunction and secondary multiple organ failure, ¹⁹ so selective embolization of the hepatic artery or embolization of the arterial supply upstream of the haemorrhagic site are satisfactory therapies for hepatic arterial bleeding. ²⁰ Embolization may effectively control bleeding, significantly improve the maternal survival rate and avoid additional trauma. In the present case, the mother underwent an emergency laparotomy, including removal of the haematoma and electrical coagulation of the wound, but the result was unsatisfactory, due to the possibility of intraoperative transient retraction of culprit vessels. Subsequently, selective hepatic artery embolization was performed and the liver haemorrhage was successfully controlled.

Conclusion

Pregnancy should not be continued in a patient with chronic kidney disease and chronic hypertension, due to risk of worsening of kidney function along with risk of superimposed preeclampsia and hepatic haemorrhage, as occurred in the present case. Both the mother and fetus survived because of round-the-clock availability of haemodialysis, ICU admission, multiple blood and blood product transfusions, early detection of hepatic haemorrhage with CT angiography, and the availability of experienced surgeons and facility to perform hepatic artery embolization. In order to prevent maternal mortality in such cases, the abovementioned specialties and superspecialist branches should be available within every institute.

Research Data

Research Data for Spontaneous hepatic haemorrhage after caesarean section in a patient with uraemia and superimposed preeclampsia: a case report

Research Data for Spontaneous hepatic haemorrhage after caesarean section in a patient with uraemia and superimposed preeclampsia: a case report by Yuanyuan Chen, Kai Liu, Kangjie Song, Changtai Fang, Lianghua Zhu, Gaofei Wu, Junjing Zha and Jiaan Zha in Journal of International Medical Research